Idiopathic Pulmonary Fibrosis: From 1 Different Sources
(IPF) a serious interstitial lung disease, formerly called cryptogenic fibrosing alveolitis (see alveolitis). It is characterized by progressive fibrous scarring of the lung and increased numbers of inflammatory cells in the alveoli and surrounding tissues. The disease is usually diagnosed on clinical grounds on a basis of worsening breathlessness, inspiratory crackles at the lung bases on auscultation, clubbing of the fingers or toes, bilateral radiographic shadowing predominantly in the lower zones of the chest X-ray, subpleural *honeycomb change on CT scanning of the chest, and restrictive lung function on spirometry. It is also called usual interstitial pneumonia (UIP; see interstitial pneumonia), a term used by lung pathologists for the most common cellular pattern seen on biopsy. Treatment includes *pirfenidone, corticosteroids, and immunosuppressants.
This is the most common serious genetic disease in Caucasian children, with an incidence of about one per 2,500 births, and more than 6,000 patients in the UK (30,000 in the USA). It is an autosomal recessive disorder of the mucus-secreting glands of the lungs, the pancreas, the mouth, and the gastrointestinal tract, as well as the sweat glands of the skin. The defective gene is sited on chromosome 7 which encodes for a protein, cystic ?brosis transmembrane conductance regulator (CFTR). Individuals who inherit the gene only on one set of chromosomes can, however, carry the defect into successive generations. Where parents have a child with cystic ?brosis, they have a one-infour chance of subsequent children having the disease. They should seek GENETIC COUNSELLING.
The disorder is characterised by failure to gain weight in spite of a good appetite, by repeated attacks of bronchitis (with BRONCHIECTASIS developing at a young age), and by the passage of loose, foul-smelling and slimy stools (faeces). AMNIOCENTESIS, which yields amniotic ?uid along with cells shed from the fetus’s skin, can be used to diagnose cystic ?brosis prenatally. The levels of various enzymes can be measured in the ?uid and are abnormal when the fetus is affected by cystic ?brosis. Neonatal screening is possible using a test on blood spots – immunoreactive trypsin (IRT).
In children with symptoms or a positive family history, the disease can be tested for by measuring sweat chloride and sodium. This detects the abnormal amount of salt that is excreted via the sweat glands when cystic ?brosis is present. Con?rmation is by genetic testing.
Treatment This consists basically of regular physiotherapy and postural drainage, antibiotics and the taking of pancreatic enzyme tablets and vitamins. Some children need STEROID treatment and all require nutritional support. The earlier treatment is started, the better the results. Whereas two decades ago, only 12 per cent of affected children survived beyond adolescence, today 75 per cent survive into adult life, and an increasing number are surviving into their 40s. Patients with end-stage disease can be treated by heart-lung transplantation (with their own heart going to another recipient). Research is underway on the possible use of GENE THERAPY to control the disorder. Parents of children with cystic ?brosis, seeking help and advice, can obtain this from the Cystic Fibrosis Trust.... cystic fibrosis
Relating to the LUNGS.... pulmonary
No known cause.... idiopathic
In this condition, increased resistance to the blood ?ow through the LUNGS occurs. This is usually the result of lung disease, and the consequence is an increase in pulmonary artery pressure and in the pressure in the right side of the heart and in the veins bringing blood to the heart. Chronic BRONCHITIS or EMPHYSEMA commonly constrict the small arteries in the lungs, thus causing pulmonary HYPERTENSION. (See also EISENMENGER SYNDROME.)... pulmonary hypertension
Fluid in the small air sacs of the lungs, from inefficient pumping by the heart or leakage of fluid from the blood vessels in the lungs (possibly from envenomation). As it prevents air exchange in the lungs it causes hypoxia and may lead to death.... pulmonary oedema
The formation of ?brous or scar tissue, which is usually due to infection, injury or surgical operation.... fibrosis
A disorder of the HEART in which obstruction of the out?ow of blood from the right ventricle occurs. Narrowing of the pulmonary valve at the exit of the right ventricle and narrowing of the pulmonary artery may cause obstruction. The condition is usually congenital, although it may be caused by RHEUMATIC FEVER. In the congenital condition, pulmonary stenosis may occur with other heart defects and is then known as Fallot’s tetralogy. Breathlessness and enlargement of the heart and eventual heart failure may be the consequence of pulmonary stenosis. Surgery is usually necessary to remove the obstruction.... pulmonary stenosis
The condition in which an embolus (see EMBOLISM), or clot, is lodged in the LUNGS. The source of the clot is usually the veins of the lower abdomen or legs, in which clot formation has occurred as a result of the occurrence of DEEP VEIN THROMBOSIS (DVT) – THROMBOPHLEBITIS (see VEINS, DISEASES OF). Thrombophlebitis, with or without pulmonary embolism, is a not uncommon complication of surgical operations, especially in older patients. This is one reason why nowadays such patients are got up out of bed as quickly as possible, or, alternatively, are encouraged to move and exercise their legs regularly in bed. Long periods of sitting, particularly when travelling, can cause DVT with the risk of pulmonary embolism. The severity of a pulmonary embolism, which is characterised by the sudden onset of pain in the chest, with or without the coughing up of blood, and a varying degree of SHOCK, depends upon the size of the clot. If large enough, it may prove immediately fatal; in other cases, immediate operation may be needed to remove the clot; whilst in less severe cases anticoagulant treatment, in the form of HEPARIN, is given to prevent extension of the clot. For some operations, such as hip-joint replacements, with a high risk of deep-vein thrombosis in the leg, heparin is given for several days postoperatively.... pulmonary embolism
A condition which may develop in both LUNGS (interstitial pulmonary ?brosis) or part of one lung. Scarring and thickening of lung tissues occur as a consequence of previous lung in?ammation, which may have been caused by PNEUMONIA or TUBERCULOSIS. Symptoms include cough and breathlessness and diagnosis is con?rmed with a chest X-ray. The patient’s underlying condition should be treated, but the damage already done to lung tissue is usually irreversible. (See also ALVEOLITIS.)... pulmonary fibrosis
See pulmonary disease, chronic obstructive.... chronic obstructive pulmonary disease
A combination of mouth to mouth resuscitation (E.A.R.) to oxygenate the blood, and external chest compression (E.C.C.) to compress the heart to help pump this artificially oxygenated blood around the body to maintain tissue oxygen concentration and prevent death.... cardio-pulmonary resuscitation
Sometimes described as thrombocytopenia, this is an autoimmune disorder in which blood PLATELETS are destroyed. This disturbs the blood’s coagulative properties (see COAGULATION) and spontaneous bleeding (PURPURA) occurs into the skin. The disease may be acute in children but most recover without treatment. Adults may develop a more serious, chronic variety which requires treatment with CORTICOSTEROIDS and sometimes SPLENECTOMY. Should the disease persist despite these treatments, intravenous immunoglobulin or immunosuppressive drugs (see IMMUNOSUPPRESSION) are worth trying. Should the bleeding be or become life-threatening, concentrates of platelets should be administered.... idiopathic thrombocytopenic purpura (itp)
Previously called juvenile rheumatoid arthritis and juvenile chronic arthritis, this is a set of related conditions of unknown cause affecting children. Characteristically, the synovial membrane of a joint or joints becomes in?amed and swollen for at leat six weeks (and often very much longer – even years). About 1 in 10,000 children develop it each year, many of whom have certain HLA genetic markers, thought to be important in determining who gets the illness. In?ammatory CYTOKINES play a big part.
Clinical features There are various types. The oligoarthritic type involves 1–4 joints (usually knee or ankle) which become hot, swollen and painful. One complication is an in?ammation of the eyes – UVEITIS. The condition often ‘burns out’, but may reappear at any time, even years later.
The polyarthritic type is more like RHEUMATOID ARTHRITIS in adults, and the child may have persistent symptoms leading to major joint deformity and crippling.
The systemic type, previously called Still’s disease, presents with a high fever and rash, enlarged liver, spleen and lymph nodes, and arthritis – although the latter may be mild. In some children the illness becomes recurrent; in others it dies down only to return as polyarthritis.
Complications These include uveitis, which can lead to loss of vision; a failure to thrive; osteoporosis (see under BONE, DISORDERS OF); joint deformity; and psychosocial diffculties.
Treatment This includes ANTIPYRETICS and ANALGESICS, including NON-STEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS), intra-articular steroid injections, anti-tumour necrosis factor drugs and steroids.
Physiotherapy is vital, and children may need to wear splints or other orthotic devices to alleviate deformity and pain. Orthopaedic operative procedures may be necessary.... juvenile idiopathic arthritis (jia)
See LUNGS, DISEASES OF.... pulmonary diseases
This is a term encompassing chronic BRONCHITIS, EMPHYSEMA, and chronic ASTHMA where the air?ow into the lungs is obstructed.
Chronic bronchitis is typi?ed by chronic productive cough for at least three months in two successive years (provided other causes such as TUBERCULOSIS, lung cancer and chronic heart failure have been excluded). The characteristics of emphysema are abnormal and permanent enlargement of the airspaces (alveoli) at the furthermost parts of the lung tissue. Rupture of alveoli occurs, resulting in the creation of air spaces with a gradual breakdown in the lung’s ability to oxygenate the blood and remove carbon dioxide from it (see LUNGS). Asthma results in in?ammation of the airways with the lining of the BRONCHIOLES becoming hypersensitive, causing them to constrict. The obstruction may spontaneously improve or do so in response to bronchodilator drugs. If an asthmatic patient’s airway-obstruction is characterised by incomplete reversibility, he or she is deemed to have a form of COPD called asthmatic bronchitis; sufferers from this disorder cannot always be readily distinguished from those people who have chronic bronchitis and/ or emphysema. Symptoms and signs of emphysema, chronic bronchitis and asthmatic bronchitis overlap, making it di?cult sometimes to make a precise diagnosis. Patients with completely reversible air?ow obstruction without the features of chronic bronchitis or emphysema, however, are considered to be suffering from asthma but not from COPD.
The incidence of COPD has been increasing, as has the death rate. In the UK around 30,000 people with COPD die annually and the disorder makes up 10 per cent of all admissions to hospital medical wards, making it a serious cause of illness and disability. The prevalence, incidence and mortality rates increase with age, and more men than women have the disorder, which is also more common in those who are socially disadvantaged.
Causes The most important cause of COPD is cigarette smoking, though only 15 per cent of smokers are likely to develop clinically signi?cant symptoms of the disorder. Smoking is believed to cause persistent airway in?ammation and upset the normal metabolic activity in the lung. Exposure to chemical impurities and dust in the atmosphere may also cause COPD.
Signs and symptoms Most patients develop in?ammation of the airways, excessive growth of mucus-secreting glands in the airways, and changes to other cells in the airways. The result is that mucus is transported less e?ectively along the airways to eventual evacuation as sputum. Small airways become obstructed and the alveoli lose their elasticity. COPD usually starts with repeated attacks of productive cough, commonly following winter colds; these attacks progressively worsen and eventually the patient develops a permanent cough. Recurrent respiratory infections, breathlessness on exertion, wheezing and tightness of the chest follow. Bloodstained and/or infected sputum are also indicative of established disease. Among the symptoms and signs of patients with advanced obstruction of air?ow in the lungs are:
RHONCHI (abnormal musical sounds heard through a STETHOSCOPE when the patient breathes out).
marked indrawing of the muscles between the ribs and development of a barrel-shaped chest.
loss of weight.
CYANOSIS in which the skin develops a blue tinge because of reduced oxygenation of blood in the blood vessels in the skin.
bounding pulse with changes in heart rhythm.
OEDEMA of the legs and arms.
decreasing mobility.
Some patients with COPD have increased ventilation of the alveoli in their lungs, but the levels of oxygen and carbon dioxide are normal so their skin colour is normal. They are, however, breathless so are dubbed ‘pink pu?ers’. Other patients have reduced alveolar ventilation which lowers their oxygen levels causing cyanosis; they also develop COR PULMONALE, a form of heart failure, and become oedematous, so are called ‘blue bloaters’.
Investigations include various tests of lung function, including the patient’s response to bronchodilator drugs. Exercise tests may help, but radiological assessment is not usually of great diagnostic value in the early stages of the disorder.
Treatment depends on how far COPD has progressed. Smoking must be stopped – also an essential preventive step in healthy individuals. Early stages are treated with bronchodilator drugs to relieve breathing symptoms. The next stage is to introduce steroids (given by inhalation). If symptoms worsen, physiotherapy – breathing exercises and postural drainage – is valuable and annual vaccination against INFLUENZA is strongly advised. If the patient develops breathlessness on mild exertion, has cyanosis, wheezing and permanent cough and tends to HYPERVENTILATION, then oxygen therapy should be considered. Antibiotic treatment is necessary if overt infection of the lungs develops.
Complications Sometimes rupture of the pulmonary bullae (thin-walled airspaces produced by the breakdown of the walls of the alveoli) may cause PNEUMOTHORAX and also exert pressure on functioning lung tissue. Respiratory failure and failure of the right side of the heart (which controls blood supply to the lungs), known as cor pulmonale, are late complications in patients whose primary problem is emphysema.
Prognosis This is related to age and to the extent of the patient’s response to bronchodilator drugs. Patients with COPD who develop raised pressure in the heart/lung circulation and subsequent heart failure (cor pulmonale) have a bad prognosis.... chronic obstructive pulmonary disease (copd)
See BELL’S PALSY.... idiopathic facial nerve palsy
Naturally produced in the LUNGS by cells called pneumocytes, this substance is a mixture of phospholipids (see PHOSPHOLIPID) and LIPOPROTEINS. Present in ?uid lining the alveoli (see ALVEOLUS) in the lungs, their action helps maintain their patency. Premature babies may have a de?ciency of surfactant, a disorder which causes severe breathing diffculties – RESPIRATORY DISTRESS SYNDROME or hyaline membrane disease – and HYPOXIA. They will need urgent respiratory support, which includes oxygen and the administration (via an endotracheal tube) of a specially prepared surfactant such as beractant (bovine lung extract) or edfosceril palmitate.... pulmonary surfactant
Cancer of the lung.
By the blood and lymph cancer may be transferred (metastasised) to the lymph nodes under the arm, liver, brain or lungs. An association has been shown between a low intake of Vitamin A and lung cancer. Causes: occupational hazards, environmental pollution, radiation, keeping of pet birds. Cigarette smoking is a strong risk factor. Studies show that a high Vitamin A/carotene intake is protective against the disease in men. Among women, evidence of a similar protective effect has not been found. Vitamin C reduces cancer risk. The increased prevalence of smoking among women results in more female lung cancer. All smokers should drink freely carrot juice (Vitamin A).
Symptoms. Chronic irritative cough, difficult breathing, pain in the chest, recurrent spitting of blood, clubbing of fingers, weight loss.
Alternatives. Only transient benefit is obtainable, yet it may be sufficient to achieve a measure of relief from distressing symptoms. See: CANCER: GENERAL REMARKS. Mullein tea has its supporters. Bugleweed strengthens lung tissue and supports the action of the heart. Blood root is known to arrest bleeding (haemoptysis).
Tea. Equal parts: Red Clover, Gota Kola, Mullein. 2 teaspoons to each cup boiling water; infuse 5-15 minutes. 1 cup three or more times daily.
Formula No 1. Equal parts: Elecampane, Violet, Red Clover, Echinacea. Mix. Dose: Powders: 750mg (three 00 capsules or half a teaspoon). Liquid extracts: 1-2 teaspoons. Tinctures: 1-3 teaspoons. Thrice daily and, if necessary, at bedtime for relief.
Formula No 2. Tincture Blood root 10 drops; Liquid extract Dogwood 20 drops; Liquid extract Elecampane 200 drops (14ml); Liquid extract Bugleweed (Lycopus europ) 30 drops. Flavour with Liquorice if necessary. Dose: 1-2 teaspoons in water 3 or more times daily. (W. Burns-Lingard MNIMH)
Where accompanied by active inflammation, anti-inflammatories are indicated: Mistletoe, Wild Yam, etc.
Diet. A substance in fish oil has been shown to experimentally prevent cancer of the lung. Mackerel, herring and sardines are among fish with the ingredient. See: DIET – CANCER.
Chinese Herbalism. See: CANCER – CHINESE PRESCRIPTION.
Treatment by a general medical practitioner or hospital oncologist. ... cancer – pulmonary
One of a large number of tiny, balloon-like sacs at the end of a bronchiole (one of many small air passages in the lungs) where gases are exchanged during respiration.... alveolus, pulmonary
(ITP) An autoimmune disorder in which platelets are destroyed, leading to bleeding beneath the skin (see purpura).... idiopathic thrombocytopaenic purpura
Scarring of lung tissue mainly involving the alveoli. There are a number of causes, including occupational exposure to dusts and fibrosing alveolitis, which is an autoimmune disorder.... interstitial pulmonary fibrosis
A rare defect of the pulmonary valve at the exit of the heart’s right ventricle. The valve fails to close properly, allowing blood to leak back into the heart. The cause is usually rheumatic fever, endocarditis, or severe pulmonary hypertension.... pulmonary incompetence
a congenital abnormality in which the pulmonary veins enter the right atrium or vena cava instead of draining into the left atrium. The clinical features are those of an *atrial septal defect.... anomalous pulmonary venous drainage
A combination of chronic bronchitis and emphysema, in which there is persistent disruption of air flow into or out of the lungs. Patients are sometimes described as either pink puffers or blue bloaters, depending on their condition. Pink puffers maintain adequate oxygen in their bloodstream through an increase in their breathing rate, and remain “pink” despite damage to the lungs. However, they suffer from almost constant shortness of breath. Blue bloaters are cyanotic (have a bluish discoloration of the skin and mucous membranes) because of obesity, and sometimes oedema, mainly due to heart failure resulting from the lung damage.... pulmonary disease, chronic obstructive
see persistent idiopathic facial pain.... chronic idiopathic facial pain
(benign intracranial hypertension, pseudotumour cerebri) a syndrome of raised pressure within the skull in the absence of a clear structural cause, such as a tumour. Although the cause is not certain, proposed mechanisms include impaired reabsorption of cerebrospinal fluid or venous outflow from the brain. The symptoms include headache, vomiting, double vision, and *papilloedema. The diagnosis is made by finding a high opening pressure at *lumbar puncture in the absence of a causative structural abnormality on brain imaging. It can improve spontaneously but drug therapy or neurosurgical treatment may be required to protect the patient’s vision.... idiopathic intracranial hypertension
(ITP) an *autoimmune disease in which platelets are destroyed, leading to spontaneous bruising (see purpura). Acute ITP is a relatively mild disease of children, who usually recover without treatment. A chronic form of the disease, typically affecting adults, is more serious, requiring treatment with corticosteroids or, if there is no response, with splenectomy. If both fail, immunosuppressant drugs may be effective. Platelet concentrates are used for life-threatening bleeding.... idiopathic thrombocytopenic purpura
(JIA, Still’s disease) any one of a group of conditions characterized by inflammation of the joints lasting longer than 6 weeks and occurring before the age of 16. The causes are unknown but immunological and infective mechanisms are suspected. JIA can affect either four or fewer joints (pauciarticular JIA) or more than four (polyarticular JIA). There are two recognized types of pauciarticular JIA: type 1, which generally affects girls below the age of four; and type 2 (juvenile-onset spondylarthropathy), which generally affects boys over the age of nine. There are also two types of polyarticular JIA, depending on the presence or absence of a particular antibody in the blood. There is a great range of severity of these diseases. Treatment consists of pain management and prevention of subsequent deformity or limitation of movement (e.g. contractures). Long-term joint damage is prevented by use of medications similar to those used in rheumatoid arthritis.... juvenile idiopathic arthritis
(NSF, nephrogenic fibrosing dermopathy) a rare condition, first reported in 1997, that occurs exclusively in patients with chronic kidney disease (CKD), who develop large areas of hardened skin with fibrotic nodules and plaques. Flexion contractures with an accompanying limitation of range of motion can also occur. Exposure to gadolinium, used as a contrast agent in magnetic resonance imaging, has been identified as a causative factor, but many patients with severe CKD have been exposed to gadolinium without consequence. Linear gadolinium preparations (Omniscan, OptiMARK) appear to carry the highest risk of NSF. There is no cure for the condition.... nephrogenic systemic fibrosis
(atypical facial pain, chronic idiopathic facial pain) a chronic pain in the face that has no known cause and does not fit the classic presentation of other cranial neuralgias. It may be stress-related, and in some cases appears to be associated with defective metabolism of *tyramine. Treatment may involve the use of antidepressants.... persistent idiopathic facial pain
the artery that conveys blood from the heart to the lungs for oxygenation: the only artery in the body containing deoxygenated blood. It leaves the right ventricle and passes upwards for 5 cm before dividing into two, one branch going to each lung. Within the lungs each pulmonary artery divides into many fine branches, which end in capillaries in the alveolar walls. See also pulmonary circulation.... pulmonary artery
(PCWP) an indirect measurement of the pressure of blood in the left atrium of the heart, which indicates the adequacy of left heart function. It is measured using a catheter wedged in the most distal segment of the pulmonary artery. See also Swan-Ganz catheter.... pulmonary capillary wedge pressure
a system of blood vessels effecting transport of blood between the heart and lungs. Deoxygenated blood leaves the right ventricle by the pulmonary artery and is carried to the alveolar capillaries of the lungs. Gaseous exchange occurs, with carbon dioxide leaving the circulation and oxygen entering. The oxygenated blood then passes into small veins leading to the pulmonary veins, which leave the lungs and return blood to the left atrium of the heart. The oxygenated blood can then be pumped around the body via the *systemic circulation.... pulmonary circulation
leakage of the pulmonary valve in the heart. Mild regurgitation is a common normal finding, but severe congenital pulmonary regurgitation may require surgical correction.... pulmonary regurgitation
see tuberculosis.... pulmonary tuberculosis
a valve in the heart lying between the right ventricle and the pulmonary artery. It is a *semilunar valve that prevents blood returning to the ventricle from the pulmonary artery.... pulmonary valve
a vein carrying oxygenated blood from the lung to the left atrium. See pulmonary circulation.... pulmonary vein
(RPF) a condition in which a dense plaque of fibrous tissue develops behind the peritoneum adjacent to the abdominal aorta. It may be secondary to malignancy, medication (methysergide, beta blockers), aortic aneurysm, or certain infections. The ureters become encased and hence obstructed, causing acute *anuria and renal failure. The obstruction can be relieved by *nephrostomy or the insertion of double J *stents. In the acute phase steroid administration may help, but in established RPF *ureterolysis is required.... retroperitoneal fibrosis
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